Department of Neurological Surgery, University of Virginia, P.O. Box 800212, Charlottesville, Virginia 22908-0212, USA.
J Clin Endocrinol Metab. 2010 Apr;95(4):1528-32. doi: 10.1210/jc.2009-2449. Epub 2010 Feb 17.
Pseudotumor cerebri has only been described after successful surgery for Cushing's disease (CD) in case reports. We sought to establish the incidence and timing of its occurrence, identify predisposing factors, characterize the clinical presentations and their severity, and examine the effects of treatment in patients who underwent surgery for CD.
This study was conducted at two tertiary care centers: The University of Virginia and the National Institutes of Health.
We conducted a retrospective review of 941 surgeries for CD (723 adults, 218 children) to identify patients who developed pseudotumor cerebri after surgery for CD and examine the associated clinical features.
Seven children (four males, three females; 3%), but no adults, developed pseudotumor cerebri postoperatively. All underwent resection of an ACTH-secreting adenoma, and postoperative serum cortisol reached a nadir of less than 2 microg/dl. After surgery, all were placed on tapering hydrocortisone replacement therapy. Within 3-52 wk, all seven patients experienced symptoms of pseudotumor cerebri and had ophthalmological examination demonstrating papilledema. One patient had diplopia from a unilateral VIth nerve palsy. Six patients were still on steroid replacement at onset of symptoms. In three patients, a lumbar puncture demonstrated elevated opening pressure. Four patients were treated successfully with a lumbar puncture, steroids, and/or Diamox. Three patients did not receive treatment, and their symptoms resolved over several months. There was no correlation between the degree of hypercortisolism (24-h urinary free cortisol) before surgery and the likelihood of developing pseudotumor cerebri after surgery (P < 0.23).
This series demonstrates a 3% occurrence of pseudotumor cerebri in children after successful surgery for CD, but the absence of the syndrome in adults. Pseudotumor cerebri manifests itself within 1 yr of surgery, often while patients are still undergoing replacement steroid therapy. A patient exhibiting signs of intracranial hypertension after surgery for CD should undergo an evaluation for pseudotumor cerebri. Recognition of the symptoms and treatment should correct and/or prevent ophthalmological sequelae.
假性脑瘤仅在库欣病（CD）手术成功后的病例报告中有所描述。我们旨在确定其发生的发生率和时间，确定易患因素，描述临床表现及其严重程度，并检查接受 CD 手术治疗的患者的治疗效果。
这项研究在两个三级护理中心进行：弗吉尼亚大学和美国国立卫生研究院。
我们对 941 例 CD 手术（723 例成人，218 例儿童）进行了回顾性审查，以确定手术后发生假性脑瘤的患者，并检查相关的临床特征。
7 例儿童（4 例男性，3 例女性；3%），但无成人，术后发生假性脑瘤。所有患者均接受 ACTH 分泌腺瘤切除术，术后血清皮质醇降至低于 2μg/dl 的最低点。手术后，所有患者均接受逐渐减少的氢化可的松替代治疗。在 3-52 周内，所有 7 例患者均出现假性脑瘤症状，并进行眼科检查显示视乳头水肿。1 例患者因单侧 VI 颅神经麻痹而出现复视。6 例患者在症状出现时仍在接受类固醇替代治疗。在 3 例患者中，腰椎穿刺显示颅内压升高。4 例患者经腰椎穿刺、类固醇和/或 Diamox 成功治疗。3 例患者未接受治疗，其症状在数月内缓解。手术前高皮质醇血症（24 小时尿游离皮质醇）的程度与手术后发生假性脑瘤的可能性之间没有相关性（P<0.23）。
本系列研究表明，CD 手术后儿童假性脑瘤的发生率为 3%，但成人中没有该综合征。假性脑瘤在手术后 1 年内表现出来，通常在患者仍接受类固醇替代治疗时。CD 手术后出现颅内压升高迹象的患者应进行假性脑瘤评估。认识到症状和治疗方法应纠正和/或预防眼科后遗症。
